Showing posts with label nevus. Show all posts
Showing posts with label nevus. Show all posts

Wednesday, July 29

Nevus lipomatosus cutaneous superficialis

  ›     ›     ›   Nevus lipomatosus cutaneous superficialis
What is nevus lipomatosus cutaneous superficialis?
Nevus lipomatosus cutaneous superficialis (NLCS) is a rare idiopathic benign hamartoma characterized by deposits of mature adipose tissue in the dermis.
NLCS was first reported by Hoffman and Zurhelle in 1921. This uncommon hamartomatous nevus manifests as soft papules appearing on the buttock, thigh or abdomen.

The nevus lipomatosus superficialis is commonly present at birth, can appear later in life. It is classified into classical and solitary forms. The classical form is characterized by clusters of pedunculated, flesh-colored or yellowish papules, nodules, or plaques which may have a smooth, wrinkled or cerebriform surface. The other very rare form of NLCS manifests as a solitary dome-shaped or sessile papule.

The nevus lipomatosus cutaneous superficialis lesions are usually unilateral and may appear in linear or zosteriform cutaneous distribution. Multiple papules of various sizes may appear simultaneously.
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They are usually static and in some cases they may progress slowly over years. Normally, NLCS papules do not recur after surgical removal. They are painless and do not discharge. These lesions are not hereditary.

Some rare coexistent anomalies

Ashish Dhamija et al. reported a rare case of classical NLCS in a 26-year-old man with bilateral cerebriform nodules over the perianal area. The patient reported occasional foul-smelling discharge from the nodules. The surface of the lesion had multiple open comedones. The patient also informed that the lesions also occurred 5 years earlier and were surgically removed. Brasanac and Boricic reported a case in which the lesion also contained dermoid cysts.

Nevus lipomatosus superficialis causes

NLCS is a rare benign idiopathic hamartomatous condition. It is an ectopic presence of mature adipose tissue in the dermis layer of skin. The epidermis is usually normal. The reticular dermis has pockets of mature adipocytes. Nevus lipomatosus cutaneous superficialis with a 2p24 gene deletion has been recently reported.

Nevus lipomatosus cutaneous superficialis signs and symptoms

The characteristics of these cutaneous lesions are:
  • single or multiple papules,
  • slow-growing,
  • asymptomatic course,
  • flesh-colored or yellowish,
  • coalescing,
  • zosteriform, linear or segmental distribution,
  • smooth or cerebriform surface,
  • classic nevus lipomatosus cutaneous superficialis occurring on pelvic girdle, the lower trunk, the gluteal region or the thigh,
  • solitary form occurring on the ear, scalp, eyelid or nose and
  • not associated with skin disorders, systemic abnormalities or malignant transformation.

Nevus lipomatosus superficialis diagnosis

A general physical examination of these cutaneous lesion will present multiple, skin-colored, pedunculated, cerebriform, soft, asymptomatic nodules of varying sizes. Skin biopsy microscopy will show the presence of clusters of mature adipose tissue in the dermis, mostly around the perivascular area, interposed among the collagen bundles.

Solitary nevus lipomatosus cutaneous superficialis may require to be differentiated from fibrolipoma, as histologically it appears similar to fibrolipoma. The differential diagnosis has to include, fibrolipoma, neurofibromatosis, nevus sebaceous, lymphangioma, neurofibroma and fibroepithelial polyp.

Nevus lipomatosus cutaneous superficialis treatment

Treatment is usually not necessary for NLCS. If there is cosmetic concern or if it is coming in the way of daily activities, surgical excision is done. Cryotherapy yields mixed results. There is a report of a case of successfully treated classic NLCS with CO2 laser. After surgery, recurrence of nevus lipomatosus cutaneous superficialis is very rare.
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References:
1.Ashish Dhamija, Ashok Meherda, Paschal D’Souza, Ram S. Meena. Nevus lipomatosus cutaneous superficialis: An unusual presentation. Indian Dermatol Online J. 2012 Sep-Dec; 3(3): 196–198.
2.Khandpur S, Nagpal SA, Chandra S, Sharma VK, Kaushal S, Safaya R. Giant nevus lipomatosus cutaneous superficialis. Indian J Dermatol Venereol Leprol. 2009;75:407–8.
3.Samia Goucha, Aida Khaled, Faten Zéglaoui, Soumeya Rammeh, Rachida Zermani, Bécima Fazaa. Nevus lipomatosus cutaneous superficialis: Report of eight cases. Dermatol Ther (Heidelb). 2011 Dec; 1(2): 25–30.
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Tuesday, May 5

Becker's nevus - Becker melanosis

  ›     ›   Becker's nevus - Becker melanosis.

What is Becker's nevus?

Becker's nevus (BN), also known as Becker's melanosis, is an unilateral, cutaneous hamartoma predominantly affecting males.
This form of pigmented nevoid melanosis was first described by American dermatologist, Samuel William Becker (1894–1964), in the year 1948. It is characterized by a large, hyperpigmented macule with irregular borders and late-set hypertrichosis.

The nevoid melanosis involved common sites are the trunk, arm, shoulder, chest, face, flank, buttock and leg. It tends to be more conspicuous in male patients, becoming apparent during adolescence. It appears to be androgen-dependent as it becomes more prominent after puberty and there is increased hairiness of the affected area. In some cases intralesional acne may develop. Researchers have also reported increased number of androgen receptors in the lesion when compared with unaffected skin.

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The frequency of Becker's pigmentary hamartoma is considered to be around 0.2% in the population. However, the prevalence can be much higher as the lesion may be less conspicuous in women and may go unnoticed and often get overlooked or misdiagnosed. These melanosis lesions are initially pale in color. They darken and become more conspicuous after sun-exposure. As the melanosis progresses, new macules develop beyond the margin of the initial patch and coalesce with it, giving a geographical contour. Fresh satellite macules may keep developing.

Becker's melanosis pathogenesis

The cause of Becker's pigmentary hamartoma is not fully understood. Though there are congenital cases, the lesion usually develops during childhood and early teenage years. Possibly a small patch of skin with the predisposition for these melanosis lesions may be present at birth. Male hormones contribute to its progression and its changes during puberty. The epidermis at the site shows mild acanthosis and hyperkeratosis with regular elongation of rete ridges.

Becker's melanosis complications

Malignant melanoma had been documented in patients with Becker's pigmentary hamartoma. Several cases of congenital BN have been reported. Book SE et al. reported a case of congenital Becker's pigmentary hamartoma with a familial association. A 16-month-old Child had a patch of melanosis on his right shoulder and upper pectoral area, extending down his arm. The patient's father also had a similar macule with hair on his left shoulder right from his childhood. The authors concluded that, "We believe this to be the first reported case of a congenital Becker's nevus with a familial association."
Becker melanosis - Becker's nevus
Becker melanosis - Becker's nevus
Sumit Kar et al. reported a 22-years-old male with coexistent Becker's nevus and type 1 neurofibromatosis. Neurofibromatosis type 1 is an autosomal dominant multisystem disorder, with cutaneous, neurologic and orthopedic manifestations. Mutation in neurofibromin gene located at chromosome 17q11.2 causes type 1 neurofibromatosis. It is a rare association of both the ailments in one individual.

George Issa et al. reported a case of giant BN and concurrent epidermal nevus (EN) in a 70 years old patient. He had a hyperpigmented scaly plaque extending along his left lateral thigh, knee and leg. "The biopsy of which demonstrates hyperkeratosis with acanthosis and increased pigmentation at the basal layer, consistent with an EN." There was also a lesion on the trunk which was diagnosed as Becker's pigmentary hamartoma.

Becker's Nevus Syndrome (BNS)

BNS is an association of nevoid melanosis with several unilateral developmental abnormalities of different organ systems such as breast hypoplasia, supernumerary nipples, aplasia of the pectoralis major muscle, mentally retardation, short stature, spina bifida, scoliosis, congenital adrenal hyperplasia, cardiac defects and an accessory scrotum.

Becker's melanosis treatment

The nevoid melanosis is generally asymptomatic and no treatment is usually necessary, except for cosmetic reasons. The hair on the melanosis patch can be removed by using epilation creams, shaving, waxing or threading. Small melanosis lesions may be surgically removed. Different laser systems have been used to treat hypertrichosis and hyperpigmentation with highly variable results. Standard acne treatments may control acne in the lesion.
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Reference:
1.Book SE, Glass AT, Laude TA. Congenital Becker's melanosis with a familial association. Pediatr Dermatol. 1997 Sep-Oct;14(5):373-5.
2.Kar S, Preetha K, Yadav N, Madke B, Gangane N. Becker's nevus with neurofibromatosis type 1. Ann Indian Acad Neurol. 2015 Jan-Mar;18(1):90-2.
3.Issa G, Blalock TW, Lesher JL. Patient with giant Becker's nevus and epidermal nevus. Dermatol Reports. 2011 Sep 12;3(2):e23.
Image source: http://en.wikipedia.org/wiki/File:Becker-Naevus.jpg
Image author: Siller
Image licence: cc-by-sa-3.0
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Sunday, April 26

Nevus sebaceous - Organoid nevus

  ›     ›   Nevus sebaceous of jadassohn - Organoid nevus.

What is sebaceous nevus?

Nevus sebaceous is a congenital hairless skin hamartoma that typically occurs on the face or scalp.
These benign skin malformations tend to be present at birth and grow commensurate with the growth of the child. These organoid lesions usually occur along the lines of Blaschko in a linear configuration. Initially the surface of the nevus may be smooth and the color may be yellow or yellowish orange. The organoid lesion thickens as the child grows and the skin becomes verrucous during adolescence.

Nevus sebaceous is also known as an "Organoid nevus" and "Nevus sebaceus of Jadassohn". The organoid lesion is an overgrowth of sebaceous glands in the particular area of the skin and hence the names. Josef Jadassohn (1863-1936) described this organoid condition in 1895. These rare lesions, also known as Jadassohn's disease II, may affect males and females of all races equally.

These lesions occur in 1 to 3 of 1000 births. In some cases the lesions may be cosmetically disturbing, requiring their surgical removal.
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It has been reported that up to 10% of patients with sebaceous organoid lesions will go on to develop further symptoms of systemic anomalies. In rare cases they may be involved with speckled lentiginous nevus with systemic anomalies or other syndromes.

Nevus sebaceous pathogenesis

Nevus sebaceous is believed to be caused by postzygotic mosaic mutations in the HRAS or KRAS genes. An abnormality of the PTCH gene has been reported in some cases of organoid lesions. Deletions in the PTCH tumor suppressor gene on chromosome 9p22.3 may be the cause of malignant transformation. There is an increased expression of androgen receptors at the lesion site leading to triggering of overgrowth after puberty.

Generally there are two stages in the development of these organoid overgrowths. The early stage is characterized by papillomatous epithelial hyperplasia. There is exophytical overgrowth of epithelial cells. The hair follicles are small and the oil glands are normal. After puberty, sebaceous and apocrine glands proliferate and become hyperplastic. The hair follicles may regress and disappear. The epidermis becomes verrucous (covered with wart-like projections).

Nevus sebaceous complications

In some patients with extensive organoid lesions, linear nevus sebaceus syndrome may be present with associated with abnormalities of the central nervous system, ophthalmologic changes, skeletal deformities and malignancy. The organoid overgrowths have been observed together with speckled lentiginous lesions which may progress into malignant conditions.

Linear nevus sebaceous syndrome (LNSS)

LNSS is characterized a large organoid lesion with a plethora of systemic abnormalities. It is a typical midline nevus, small in size at birth and enlarged at puberty with verrucous epidermis with possibility of malignancy, especially basal cell epithelioma. Several organ systems are involved in this rare organoid syndrome. The syndrome is believed to be caused by an autosomal dominant lethal mutation that survives by somatic mosaicism (Gorlin et al., 2001).
image of organoid nevus sebaceous of Jadassohn
image of organoid nevus sebaceous of Jadassohn
The patient may affected by several disorders including seizures, intellectual deficit, aortic coarctation (narrowing of the aorta), skeletal dysplasia (dwarfism), retinal anomalies and urogenital anomalies. Early multidisciplinary care is recommended including removal of the organoid nevus followed by orthopedic and ophthalmic care.

Phakomatosis pigmentokeratotica

The organoid sebaceous lesion is sometimes present in association with nevus spilus and is known as phacomatosis pigmentokeratotica. These may be other extracutaneous anomalies associated with the organoid nevus syndrome. Hemiatrophy (one side of the body under-developed), muscle weakness and sensory nerve abnormalities are some of the symptoms associated with phakomatosis pigmentokeratotica.

It is presumed to be caused by twin spotting genetic mechanism, which is a specific form of somatic recombination of two genetically different clones of neighboring cells in a background of normal cells. As there is the possibility of phakomatosis pigmentokeratotica organoid lesions turning malignant, the patient must be closely monitored to ensure early detection and treatment of malignancy.

Nevus sebaceous and malignancy

In rare cases, after puberty, nevus sebaceous of Jadassohn is known to give rise to benign tumors such as trichoblastoma, sebaceoma and syringocystadenoma papilliferum. Malignant neoplasms, including linear squamous cell papilloma and malignancies such as basal cell epithelioma, basal cell carcinoma or sebaceous carcinoma may also develop. Rapid, circumscribed enlargement, ulceration or development of an exophytic nodule should raise suspicion of malignant transformation.

Izumi M. et al. reported on their study of ten cases of sebaceous carcinoma arising in nevus sebaceus. The organoid lesions predominantly occurred on the scalp of elderly women with several pathological features of sebaceous carcinoma. They reported that "all cases were treated by excision of the malignant lesion, with or without inclusion of the nevus sebaceus. In a follow-up period of 1-7 years, there was no case of recurrence".

It is generally assumed that rare malignant conditions in the organoid lesions occur after puberty. However there are nine documented cases of basal cell carcinoma arising in organoid lesions in childhood. Jensen AL. et al. reported a case of an 8-year-old male child with nevus sebaceus and facial trichoepitheliomas who developed a basal cell carcinoma. Altaykan A. et al. reported, "few cases of basal cell carcinoma arising in nevus sebaceous before puberty were reported. Herein, we report a case of basal cell carcinoma arising in a nevus sebaceous on the scalp of a 10-year-old boy".

Treatment and management of organoid lesions

Patients with extensive linear lesions should be appropriately diagnosed and evaluated to rule out malignant conditions and systemic involvement. If the sebaceous lesion is small and not cosmetically disfiguring, it may require only periodic clinical evaluation.

Prophylactic surgical removal may be considered if the organoid growth may give rise to cosmetic problems in the future. In some patients, the surgical removal may not be feasible, considering the extent of the lesion and the areas affected. Other nonsurgical treatment modalities such as carbon dioxide laser therapy and photodynamic therapy (photochemotherapy) along with topical applications have been attempted with varying degrees of success. However these procedures may impede the detection and diagnosis of malignancy in the organoid nevus sebaceous of Jadassohn.
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Reference:
1.http://omim.org/entry/163200
2.Izumi M, Tang X, Chiu CS, Nagai T, Matsubayashi J, Iwaya K, Umemura S, Tsuboi R, Mukai K. Ten cases of sebaceous carcinoma arising in nevus sebaceus. J Dermatol. 2008 Nov;35(11):704-11.
3.Santibanez-Gallerani A, Marshall D, Duarte AM, Melnick SJ, Thaller S. Should nevus sebaceus of Jadassohn in children be excised? A study of 757 cases, and literature review. J Craniofac Surg. 2003 Sep;14(5):658-60.
4.Altaykan A, Ersoy-Evans S, Erkin G, Ozkaya O. Basal cell carcinoma arising in nevus sebaceous during childhood. Pediatr Dermatol. 2008 Nov-Dec;25(6):616-9.
Image source: http://en.wikipedia.org/wiki/File:NaevusSebaceus_res.jpg
Image author: Phrontis
Image licence: cc-by-sa-3.0
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Monday, April 20

Hori's nevus - ABNOM

  ›     ›   Hori's nevus - Acquired bilateral nevus of Ota-like macules (ABNOM).

What is Hori's nevus (ABNOM)?

Hori's nevus (acquired bilateral nevus of Ota-like macules or ABNOM) is a hamartoma of dermal melanocytes.
ABNOM were first described by Hori et al. in 1984. ABNOM acquired this name because of their similarities to nevus of Ota. ABNOM are predominantly found in female patients who are over thirty years of age and more. ABNOM lesions are prevalent in people of Asian origin. Bei-Qing Wang et al. in a study in Shanghai, China, found that "the overall prevalence rate of ABNOM in Shanghai was 2.5 and 4.2% in females."

ABNOM is an acquired form of dermal hyperpigmentation on the face. Hori's nevus clinically present as multiple, speckled, bilateral, blue-brown and/or slate-gray lesion occurring on the face. ABNOM are more commonly observed on the malar regions (cheek bone) and less commonly on forehead, upper eyelids, cheeks and nose. Normally in ABNOM no ocular or mucosal involvement is seen. Histopathologic studies support the view that ABNOM is a separate entity from Ota lesions.
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ABNOM causes

The pathogenesis of ABNOM is not known. One theory proposes the cause of Hori's nevus as dropping-off of epidermal melanocytes in the basal layer of epidermis into dermal layers. Another view proposes activation of preexisting latent dermal melanocytes by some triggering factor into ABNOM. Some researchers attribute an association with female hormones as the prevalence of Hori's macules is higher in women during their reproductive age. In another study by Long et al., it was found that the expression of androgen receptors increase in ABNOM lesions, whereas estrogen and progesterone receptors expression do not increase.

ABNOM diagnosis

Hori's macules are not present at birth. ABNOM may initially present be as discrete brown patches. Hori'S nevus may progress with time to acquire slate-grey color. Hori's macules have irregularly shaped bipolar melanocytes with many singly dispersed melanosomes. In Hori's nevus, the melanocytes are dispersed in the middle and the upper dermis and the skin structure is not disturbed. Melanocytes in ABNOM are dispersed in the perivascular areas of dermis.

ABNOM abnormal manifestations

Hori's macules in some rare cases may involve mucous membranes. Ramesh M Bhat et al reported a case of ABNOM with a mucosal involvement in a 42 year old South Indian male patient. He had "multiple, discrete, speckled, blue-gray to slate-gray, hyperpigmented macules, symmetrically distributed on either side of the face, involving the forehead, anterior aspect of the scalp, bilateral temporal, zygomatic, malar areas, upper eyelids, nose, and pinnae. Blue-brown discoloration of both sclera and a hyperpigmented irregular mottled patch over the hard palate was also noted." Based on clinical and histopathological findings it was diagnosed as Hori's macules.

In some rare cases both Ota and Hori's macules can be present in the same patient. Jung-Hun Park et al reported a case of simultaneous presence of Ota macules and ABNOM in a 36-yr-old Korean woman. She had dark bluish lesion on her right cheek and right conjunctiva since birth. She also had mottled brownish macules on both sides of forehead and both sides of lower eyelids that have developed three years ago. With histopathological studies the team diagnosed the lesion on the right cheek as nevus of Ota and and those macules on the forehead and eyelids as ABNOM.

ABNOM treatment

ABNOM has been successfully treated with pigment-specific lasers including the Q-switched Nd: Yag laser (1064 nm), Q-switched alexandrite laser (755 nm), and Q-switched ruby laser (694 nm). The concurrent use of QS 532nm Nd:YAG in combination with the QS 1064nm Nd:YAG laser had also given successful results in treating Hori's nevus.
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Reference:
Reference: 1.Park JH, Lee MH. Acquired, bilateral nevus of Ota-like macules (ABNOM) associated with Ota's nevus: case report. J Korean Med Sci. 2004 Aug;19(4):616-8.
2.Park JM, Tsao H, Tsao S. Acquired bilateral nevus of Ota-like macules (Hori nevus): etiologic and therapeutic considerations. J Am Acad Dermatol. 2009 Jul;61(1):88-93.
3.Bei-Qing Wang, Zheng-Yu Shen, Ye Fei, Hong Li, Jian-Hang Liu, Hui Xu, Zhen Zhang, Xiao-Hong Yu, Xiang-Dong Chen. A Population-Based Study of Acquired Bilateral Nevus-of-Ota-Like Macules (ABNOM) in Shanghai, China. Journal of Investigative Dermatology (2011) 131, 358–362.
4.Polnikorn, N., Tanrattanakorn, S., Goldberg, D. J. (2000). Treatment of Hori's Nevus with the Q-Switched Nd:YAG Laser. Dermatologic Surgery, 26: 477–480.
5.Bhat RM, Pinto HP, Dandekeri S, Ambil SM. Acquired bilateral nevus of ota-like macules (ABNOM) with Mucosal involvement: A new variant of Hori's nevus. Indian J Dermatol 2014;59:293-6
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Current topic in natural skin care: Hori's nevus - Acquired bilateral nevus of Ota-like macules (ABNOM).

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Thursday, April 16

Nevus flammeus - Port wine stain birthmark

  ›     ›   Nevus flammeus - Port wine stain birthmark.

What is nevus flammeus?

Nevus flammeus (also known as Port wine stain) is a vascular birthmark.
Nevus flammeus birthmark appears like a stain caused by spilling of port wine. Nevus flammeus is congenital and is usually present at birth and in a few cases it may appear in the early weeks of life. Port wine stain birthmarks grow in size commensurate with the growth of the child. These birthmarks usually express unilaterally (one side of the body) and do not cross the midline. The incidence of nevus flammeus birthmarks in newborns is estimated to be in the order of 0.3% to 0.5%.

Port wine stain is a lateral telangiectatic nevus. Unlike salmon patch, this flammeus birthmark is a rare finding in newborns and usually persists throughout the life of the individual. Though these birthmarks can appear anywhere on the body, they occur more often on the face and then on neck and upper trunk. Initially, the port wine stain appears as flat and pink lesion. As the child grows, the birthmarks deepen in color to dark red or purple. In adults the nevus flammeus birthmarks thicken and become uneven and lumpy.
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A few cases of acquired port wine stains appearing in adults had been reported. Port-wine stain birthmarks may be part of a syndrome such as Sturge–Weber syndrome or Klippel–Trénaunay–Weber syndrome. In some cases these birthmarks have been attributed to c.548G→A mutation in the GNAQ gene. Some researchers described an association with RAS p21 protein activator 1 (RASA1), a 120-kDa cytosolic human protein. The increase in the presence of vascular endothelial growth factor (VEGF)–A and its most active receptor VEGF-R2 may be contributing to the vessel proliferation and/or vasodilatation.

Nevus flammeus causes

The nevus flammeus birthmark is caused by defective vasculature of the skin. Primarily, the vasculature of the superficial plexus in the dermal skin shows enlargement or dilation of capillaries. The cutaneous sympathetic innervation in the perivascular region brings about vasoactive responses to both vasodilating and vasoconstricting stimuli. Inadequate innervation in nevus flammeus birthmark area may be responsible for decreased vascular tone and progressive vascular dilatation.

In case of salmon patch (nevus simplex), there appears to be a defect/delay in maturation of cutaneous sympathetic innervation, which clears up as the infant grows. However, in the case of nevus flammeus birthmark, there is loss of associated peripheral nervous elements in the affected perivascular region. Such condition leads to reduced vasoactive responses to vasodilating and vasoconstricting stimuli and permanent dilation of capillaries. There may be fluid accumulation and cellular infiltration in the perivascular region of port wine stain birthmarks leading to uneven skin.

Nevus flammeus diagnosis

Port wine stain birthmarks are usually diagnosed based entirely upon the history and appearance. In some cases skin biopsy may be performed to confirm the diagnosis. The birthmarks near eye or on the eyelid may require a test of the ocular pressures in that eye to rule out complications such as vision problems, glaucoma or blindness as a swollen port wine stain birthmark may create pressure on the eye. In infants with nevus flammeus birthmarks on the head or forehead, a MRI scan study of the brain may be necessary to rule out Sturge-Weber syndrome.
Image of port wine stain birthmark
Image of nevus flammeus birthmark
Port wine stain birthmarks can be easily differentiated from other vascular birthmarks like hemangioma and nevus simplex. Skin hemangiomas appear as raised swellings and have a rapid growth phase followed by an involution phase. Salmon patch birthmarks are pink in color and appear flat and irregularly shaped. Salmon patches in most cases fade and disappear by two years. Though nevus flammeus birthmarks initially appear flat and pink, they do not fade and persist throughout the life. Over time, port-wine stains become dark red or purple in color, thicken, become uneven and show hypertrophy.

Port wine stain associated syndromes

Nevus flammeus birthmark is a cutaneous finding of several syndromes including Sturge-Weber syndrome, Klippel-Trenaunay syndrome, Parkes-Weber syndrome and Proteus syndrome.

Nevus flammeus and carcinoma

Sirunya Silapunt et al. reported a case of basal cell carcinoma arising in a nevus flammeus birthmark in Dermatologic Surgery. A few cases of basal cell carcinoma developing in a port-wine stain birthmark have been reported in medical literature. The authors detected a nodular basal cell carcinoma on the margin of a previously untreated port-wine stain on the left cheek of an 87-year-old patient. The carcinoma was successfully excised and there was no evidence of recurrence of the tumor in the birthmark after twelve months. They concluded that,
"basal cell carcinoma should be included in the differential diagnosis of a skin lesion occurring in a port-wine stain."

Nevus flammeus treatment

In the absence of successful treatment, increased tissue mass (hypertrophy), thickening and appearance of lumps in nevus flammeus birthmark may cause cosmetic, emotional and social problems and increasing disfigurement. The birthmarks near the eyelids and lips may impair their functions later in life. The treatment has to be started before one year of age. Early treatment, before thickening and hardening of the port wine stain birthmarks gives better results.

A number of treatment options have been used with limited success in treating port wine stain birthmarks. Flashlamp-pumped pulsed-dye laser (PDL) surgery is considered as a treatment of choice for nevus flammeus birthmarks. PDL causes selective destruction of the capillary malformations by selective photothermolysis. PDL induces intravascular coagulation and rupture of smaller vessels in the nevus. Later the photothermolysis mass of cells in the port wine stain are absorbed and replaced by collagen.
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Reference:
1.Pasyk KA. Familial multiple lateral telangiectatic nevus (port-wine stain or nevus flammeus). Clin Genet. 1992 Apr;41(4):197-201.
2.Gao XH, Wang LL, Zhang L, Hong YX, Wei H, Chen HD. Familial nevus flammeus associated with early onset cherry angiomas. Int J Dermatol. 2008 Dec;47(12):1284-6.
3.Faurschou A, Olesen AB, Leonardi-Bee J, Haedersdal M. Lasers or light sources for treating port-wine stains. Cochrane Database Syst Rev. 2011 Nov 9;(11):CD007152.
4.Silapunt S, Goldberg LH, Thurber M, Friedman PM. Basal cell carcinoma arising in a port-wine stain. Dermatol Surg. 2004 Sep;30(9):1241-5.
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Current topic in natural skin care: Nevus flammeus - Port wine stain birthmark.

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Sunday, April 12

Nevus spilus - Speckled lentiginous nevus

  ›     ›   Nevus spilus (speckled lentiginous nevus).
What is nevus spilus?
Nevus spilus (aka speckled lentiginous nevus) is a common cutaneous lesion.
It is characterized by multiple small dark hyperpigmented macules or papules superimposed on tan-brown, café au lait spot-like pigmented area. Though it may be present at the time of birth, the speckled lentiginous nevus (SLN) usually develops during the early childhood. The term spilus is derived from the Greek word 'spilos' meaning 'spot'. Cohen et al. in 1970 described the nevus spilus (NS) lesions as
"a circumscribed tan macule in which more darkly pigmented, raised, and/or flat melanocytic or nevomelanocytic elements are distributed."

Clinical presentation

The speckled lentiginous lesion is a lentigo simplex macule superimposed with melanocytic nevus. Though the NS may appear anywhere on the body, its common locations are chest and upper limbs. Quite often the arrangement of the spilus lesion follows the lines of Blaschko.
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The speckled lentiginous lesion may either be papular or macular and sometimes found in association with some specific systemic syndrome. The speckles are often macular rather than papular. The spots may be pigmented dark and appear as black, brown or red-brown macules and papules.

The speckled lentiginous macules can be classified into three distinct clinical types. The macule is considered small, if it is less than 1.5 cm, medium if it is between 1.5 to 19.9 cm and large or giant if it exceeds 20 cm. There also exists segmental distribution of speckled lentiginous lesion, known as zosteriform nevus spilus. The number of speckles differs generally in relation to size of the NS and in some cases up to 30 speckles have been recorded. The speckles usually range between one to three millimeters, though larger sizes have been recorded.

Histopathology and diagnosis of speckled lentiginous nevus

The early spilus lesion in its non-spotted form appears similar to café au lait spot. Though the tan background of speckled lentiginous lesions appears similar to the café au lait spot, they are separate entities clinically and histologically. The speckled lentiginous lesions consistently have been shown to contain nevus cells, which are large variations of typical melanocytes, lacking dendrites and having more abundant cytoplasm with coarse pigment granules. Though the presence of nevus cells in the café au lait spot had been recorded in some studies, there are no records of the presence of speckles within these café au lait spots.

The speckled lentiginous nevus has similarity in appearance to agminated lentigines. Agminated lentigines may be present at birth or form in early childhood. Agminated lentiginosis is an unusual pigmentary disorder, characterized by numerous pigmented spots grouped within an area of normal skin. The distinguishing feature of agminated lentiginosis is that it appears on a normal skin whereas speckled lentiginous spilus appears on a tan-brown skin.

Histologically, the light tan-brown background patch of nevus usually shows mild hyperplasia of melanocytes. The speckled lentiginous spilus also shows features of lentigo simplex, including the elongation of rete ridges (epidermal thickenings that extend downward between dermal papillae) and increase in the concentration of basal layer melanocytes. Nevus spilus also shows increase in melanin concentration and number of melanosomes present in the melanocytes, basal keratinocytes and dermal melanophages. Junctional nests of melanocytes are seen in the hyperpigmented speckled lentiginous spilus spots.

Malignant melanoma and nevus spilus

In very rare cases the speckled lentiginous spilus had turn into a malignant melanoma. Maria Teresa Corradin et al. reported a case of a pre-existing spilus in NS turning into malignant melanoma in an 80-year-old male patient. They inferred that
"The risk factor of malignant transformation seems to increase when the lesion is congenital or acquired in infancy, when its size is ≥ 4 cm, and when the type of NS is macular rather than papular".
There are recent observations of malignant melanoma developing within the lesions and a few fatal cases have been reported. The risk of developing melanoma in the NS is increased if NS is present at birth, is more than 4 cm, is of giant form or is of zosteriform.

Speckled lentiginous nevus and complications

  • FACES syndrome is a syndrome of unique facial features, anorexia, cachexia, eye and skin anomalies. Some cases of speckled lentiginous spots are associated with FACES syndrome.
  • The birth defect, phacomatosis pigmentovascularis is associated with macular nevus spilus.
  • The complex birth defect phacomatosis pigmentokeratotica is associated with papular speckled lentiginous spots.
  • "Speckled lentiginous nevus syndrome, a recently recognized phenotype is characterized by a speckled lentiginous nevus of the papular type and ipsilateral neurological abnormalities in the form of dysesthesia, muscular weakness or hyperhidrosis".

Nevus spilus treatment and management

Cases of nevus spilus transformation into melanoma requires excision to prevent recurrence and eliminate the source fields. However the aggressive treatment may leave a permanent scar. So this option may be exercised when there is clear evidence of melanoma. The Q-switched ruby or Q-switched Nd:YAG lasers are useful in treating both the background hyperpigmentation and the hyperpigmented spots. Treating nevus spilus with laser therapy had showed good clinical clearance with enhanced cosmetic effect. But the threat of speckled lentiginous lesions transforming into melanoma always exists, requiring periodic evaluation.
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Reference:
1.Vidaurri-de la Cruz H, Happle R. Two distinct types of speckled lentiginous nevi characterized by macular versus papular speckles. Dermatology. 2006;212:53-58.
2.Abecassis S, Spatz A, Cazeneuve C, et al. Melanoma within nevus spilus: 5 cases [in French]. Ann Dermatol Venereol. 2006;133:323-328.
3.Kar H, Gupta L. Treatment of nevus spilus with Q switched Nd:YAG laser. Indian J Dermatol Venereol Leprol 2013;79:243-5.
4.Vente C, Neumann C, Bertsch H, Rupprecht R, Happle R. Speckled lentiginous nevus syndrome: report of a further case. Dermatology. 2004;209(3):228-9.
5.Vaidya DC, Schwartz RA, Janniger CK. Nevus spilus. Cutis. 2007 Dec;80(6):465-8.
6.Corradin, M. T., Giulioni, E., Fiorentino, R., Santeufemia, D. A., Re, G. L., & Vettorello, A. (2014). In situ malignant melanoma on nevus spilus in an elderly patient. Acta dermatovenerologica Alpina, Panonica, et Adriatica, 23(1), 17-19.
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